Splenectomy for people with thalassaemia major or intermedia.
CONCLUSIONS: The review was unable to find good quality evidence, in the form of randomised controlled studies, regarding the efficacy of splenectomy for treating thalassaemia major or intermedia. The single included study provided little information about the efficacy of splenectomy, and compared open surgery and laparoscopic methods. Further studies need to evaluate the long-term effectiveness of splenectomy and the comparative advantages of surgical methods. Due to a lack of high quality evidence from randomised controlled studies, well-conducted observational studies may be used to answer this question. PMID: 31529486 [PubMed - as supplied by publisher]
ConclusionsFocusing on how to cope with future episodes of hemoptysis and the associated anxiety can be helpful to patients. Proactive communication and sensitivity to patient experience may deepen physician ‐patient rapport, increase self‐efficacy to cope with future episodes and lead to more comprehensive care of hemoptysis.
The severe form of CF liver disease, multilobular biliary cirrhosis (MBC) is associated with the almost invariable development of portal hypertension (PH) and the complications of variceal bleeding [1 –4] and unusually hepatic encephalopathy  or liver failure requiring transplantation . These features in combination with splenomegaly and laboratory evidence of hypersplenism facilitate a diagnosis of MBC/PH which can be confirmed with non-invasive ultrasonography [2,7–9] and magnetic res onance imaging.
In this study, the full-length PcrV was divided into four domains with the guidance of its structure, and the Nter domain (Met1-Lys127) and H12 domain (Leu251-Ile294) were found to be immunodominant. Subsequently, Nter and H12 were combined with a flexible linker to generate an artificial PcrV derivative (PcrVNH). PcrVNH was successfully produced in E. coli and behaved as a homogenous monomer. Moreover, immunization with PcrVNH elicited a multifactorial immune response and conferred broad protection in an acute PA pneumonia model and was equally effective to full-length PcrV. In addition, passive immunization with anti-Pcr...
CONCLUSIONS: Despite the discovery of a beneficial effect of systemically administered tranexamic acid and EACA in preventing postoperative bleeding in people with haemophilia undergoing dental extraction, the limited number of randomised controlled trials identified, in combination with the small sample sizes and heterogeneity regarding standard therapy and treatment regimens between the two trials, do not allow us to conclude definite efficacy of antifibrinolytic therapy in oral or dental procedures in people with haemophilia. No trials were identified in people with VWD. PMID: 31002742 [PubMed - as supplied by publisher]
Abstract Cystic fibrosis liver disease (CFLD) remains the third leading cause of death in patients with cystic fibrosis. Although most patients with CFLD present in childhood, recent studies suggest a second wave of liver disease in adulthood. There are no clear guidelines for diagnosing CFLD. Treatment options for CFLD remain limited, and while UDCA is widely used, its long-term benefit is unclear. Those who develop hepatic decompensation or uncontrolled variceal bleeding may benefit from liver transplant, either alone, or in combination with lung transplant. PMID: 30947876 [PubMed - in process]
Cystic fibrosis liver disease (CFLD) remains the third leading cause of death in patients with cystic fibrosis. Although most patients with CFLD present in childhood, recent studies suggest a second wave of liver disease in adulthood. There are no clear guidelines for diagnosing CFLD. Treatment options for CFLD remain limited, and while UDCA is widely used, its long-term benefit is unclear. Those who develop hepatic decompensation or uncontrolled variceal bleeding may benefit from liver transplant, either alone, or in combination with lung transplant.
CONCLUSIONS: A pathway utilizing systemic antifibrinolytic therapy to treat hemoptysis in CF patients was associated with a reduction in hospital admissions. No serious adverse events were observed. Additional studies are needed to further define the benefits of systemic antifibrinolytic use in CF patients. PMID: 30790551 [PubMed - as supplied by publisher]
CONCLUSIONS: No randomised controlled trials were identified investigating the relative effectiveness of DDAVP for bleeding during pregnancy in women with congenital bleeding disorders. In the absence of high-quality evidence, clinicians need to use their clinical judgement and lower level evidence (e.g. from observational trials) to decide whether or not to treat women with congenital bleeding disorders with DDAVP.Given the ethical considerations, future randomised controlled trials are unlikely. However, other high-quality controlled studies (such as risk allocation designs, sequential design, parallel cohort design) to ...
Publication date: February 2019Source: Journal of Vascular and Interventional Radiology, Volume 30, Issue 2Author(s): Frederic Bertino, C. Matthew Hawkins, Giri Shivaram, Anne E. Gill, Matthew P. Lungren, Aaron Reposar, Daniel Y. Sze, Gloria L. Hwang, Kevin Koo, Eric MonroeAbstractPurposeTo examine the technical feasibility and clinical efficacy of transjugular intrahepatic portosystemic shunt (TIPS) creation in children and adolescents.Materials and MethodsRetrospective review was performed of 59 patients (mean age 12.6 y [range, 1.5–20 y], mean weight 47.5 kg [range, 11.4–112.2 kg], mean Model for End-stage L...
This article explores the need for blood sampling in neonates, discusses the procedure and outlines the types of lancets available. It describes the Neoheel Safety Lancet (Smiths Medical), whose features are designed to avoid pain and trauma during the procedure. Three case studies are included to describe its use in clinical practice. PMID: 30673311 [PubMed - in process]